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By Karl R. White, Utah State University, Logan, Utah

Editor's note: In Texas we have made great strides implementing newborn infant hearing screening as a result of The Newborn Infant Hearing Screening and Intervention Act of 1999. We thought you might be interested in knowing more about the importance of early hearing loss detection and intervention, as this program, like many others, will be scrutinized when budgets are drawn up in Washington DC and in Texas. If you have questions or would like to know more about this issue, please contact Karl White in Logan, Utah, at .

Background

Hearing loss continues to be the most common birth defect in America. The Newborn Infant Hearing Screening and Intervention Act of 1999 (as introduced by Representative James Walsh), was incorporated as Title VI of the Labor, HHS and Education Appropriations Act of 1999, and signed into law. This law has enabled federal funds for state grants to develop infant hearing screening and intervention programs. Congress also identified several specific goals to address the problem of hearing loss in children through the Children's Health Act of 2000 (P.L. 106-310), including early hearing screening and evaluation of all newborns, coordinated intervention and rehabilitation services, and ongoing applied research to better understand the learning and developmental needs of deaf or hard-of-hearing children.

Since 1999, the number of states requiring statewide newborn hearing screening by law or voluntary compliance has increased from 11 to 41 states and the District of Columbia. While progress is being made, there is still a long way to go. Only 67% of babies are now screened for hearing loss before 1 month of age (up from only 20% in 1999). To date, over half of the newborns who do not pass the hearing screening are lost to follow-up. Moreover, over half of the infants diagnosed with hearing loss are not enrolled in early intervention programs by 6 months of age.

Over the last three years, there has been more grant applications than funding available, which underscores the need to extend federal funding. Currently, 44 states and 3 territories (out of 50 states and 9 territories) have received HRSA competitive grants for the purpose of implementing statewide EHDI programs. Since these grants have only been operational for 6 months to 2 years, a dedicated source of funding is critical at this time to ensure that state programs become fully operational, successful and properly link screening programs with diagnosis, early intervention and the child's medical home.

Only 30 states have received CDC cooperate agreement grants over the last two years (15 states in FY2000 and another 15 states in FY2001) to assist them in developing strong surveillance and tracking systems. These systems are needed to ensure follow-up and coordination of early intervention services for young children identified with hearing loss. States also face multiple challenges in transferring information about children diagnosed with hearing loss among service providers, the state EHDI programs and early intervention programs.

Facts on Hearing Loss in Children

Everyday in the United States, approximately 1 in 1,000 newborns (or 33 babies every day) is born profoundly deaf with another 2-3 out of 1,000 babies born with partial hearing loss, making hearing loss the number one birth defect in America. (National Center on Hearing Assessment & Management website 2002, Centers for Disease Control website, 2002)

Newborn hearing loss is 20 times more prevalent than phenylketonuria (PKU), a condition for which all newborns are currently screened. (Grosse, 2001).

Of the 12,000 babies in the United States born annually with some form of hearing loss, only half exhibit a risk factor _ meaning that if only high-risk infants are screened, half of the infants with some form of hearing loss will not be tested and identified. (Harrison & Roush, 1996) In actual implementation, risk-based newborn hearing screening programs identify only 10-20% of infants with hearing loss. (Elssmann, Matkin, & Sabo, 1987) When hearing loss is detected beyond the first few months of life, the most critical time for stimulating the auditory pathways to hearing centers of the brain is lost, significantly delaying speech and language development.

Only 67% of babies are now screened for hearing loss before 1 month of age (up from only 20% in 1999). Of the babies screened, only 56% who needed diagnostic evaluations actually receive them by 3 months of age. Moreover, only 53% of those diagnosed with hearing loss are enrolled in early intervention programs by 6 months of age. (National Center on Hearing Assessment website, 2002) As a result, these children tend to later re-emerge in our schools' special education (IDEA, Part B) programs.

When children are not identified and do not receive early intervention, special education for a child with hearing loss costs schools an additional $420,000, and has a lifetime cost of approximately $1 million per individual. (Johnson et al, 1993)

National Recommendations on Early Hearing Detection & Intervention

The Joint Committee on Infant Hearing (Joint Committee on Infant Hearing, 2000) and U.S. Public Health Service's Healthy People 2010 health objectives (Healthy People 2010 website, 2002) recommend that all newborns be screened for hearing loss by 1 month of age, have diagnostic follow-up by 3 months, and receive appropriate intervention services by 6 months of age.

A National Institutes of Health (NIH) Consensus Panel in 1993 recommended hearing screening of all newborns. The consensus report concluded that the best opportunity for achieving this goal is provided by the development of hearing screening programs for newborns in hospital nurseries or in birthing centers, prior to discharge. (National Institutes of Health, 1993)

The U.S. Preventive Services Task Force in 2001 concluded that universal newborn hearing screening does lead to earlier identification and treatment. However, there were not enough clinical studies of sufficient size and strength to evaluate long-term outcomes. While the preponderance of anecdotal evidence and clinical research indicates that EHDI provides substantial benefit, additional clinical outcome studies and clinical trials are needed. (Agency for Healthcare Research and Quality website, 2002)

Methods and Costs for Newborn Hearing Screening

Advances in technology for newborn hearing screening at most birthing hospitals have allowed for cost containment, with current charges ranging from $25 to $60. The cost of identifying a newborn with hearing loss is less than one-tenth the cost of identifying newborns with PKU, hypothyroidism, or sickle cell anemia, which are screened for in nearly every state. (Grosse, 2001)

Two types of electrophysiologic procedures are used to screen newborns singly or in combination:

Auditory brainstem responses (ABR) are measured by placing sensors on the baby's head. Sound is then introduced to the baby's ears through tiny earphones while the child sleeps. A computer allows brainwave activity to be recorded to indicate whether the ear and auditory brainstem pathway are responding to sound. This test is painless and takes only about 5 minutes.

Otoacoustic emissions (OAE) are faint sounds produced by most normal inner ears. The sounds cannot be heard by people, but can be detected by very sensitive microphones that are placed in the ear canal. During testing, a tiny flexible plug is inserted into the baby's ear and sound is then projected into the ear through the plug. A microphone inside the plug records the otoacoustic emissions that the normal ear produces in response to the incoming sound. Testing is also painless, takes about 5 minutes to complete, and can be done while the baby sleeps.

Benefits of Early Hearing Detection and Intervention

Infants identified with hearing loss can be fit with amplification by as young as 4 weeks of age. With appropriate early intervention, children with hearing loss can be mainstreamed in regular elementary and secondary education classrooms. (Joint Committee on Infant Hearing, 2000) Recent research has concluded that children born with a hearing loss who are identified and given appropriate intervention before 6 months of age demonstrated significantly better speech and reading comprehension than children identified after 6 months of age. (Yoshinaga-Itano & Apuzzo, 1998 and Yoshinago-Itano et al, 1998)

Even mild hearing loss can significantly interfere with the reception of spoken language and education performance. Research indicates that children with unilateral hearing loss (in one ear) are ten times as likely to be held back at least one grade compared to children with normal hearing. (Bess, 1985, Bess, 1998, and Oyler et al, 1988) Similar academic achievement lags have been reported for children with even slight hearing loss. (Quigley, 1978) Children with mild hearing loss miss 25-50% of speech in the classroom and may be inappropriately labeled as having a behavior problem. (Flexer, 1994)

Recent clinical studies indicate that early detection of hearing loss followed with appropriate intervention minimizes the need for extensive habilitation during the school years and therefore reduces the burden on the IDEA Part B program. (Centers for Disease Control and Prevention website 2002 and Ross 2001) In contrast, a 30-year Gallaudet study revealed that half of the children with hearing loss graduate from high school with a 4th grade reading level or less. (Gallaudet Research Institute website, 2002)

Centers for Disease Control and Prevention. National Center for Birth Defects and Developmental Disabilities, Early Hearing Detection and Intervention Program. What is EHDI?http://www.cdc.gov/ncbddd/ehdi/ehdi.htm.

Grosse, S. Cost comparison of screening newborns for hearing impairment and biochemical disorders. Centers for Disease Control and Prevention. Paper presented at the Newborn Screening and Genetics Conference, May 2001.

Harrison, M and Roush, J. Age of suspicion, identification and intervention for infants and young children with hearing loss: a national study. Ear and Hearing. 1996; 17:55-62.

Joint Committee on Infant Hearing. Year 2000 position statement: principles and guidelines for early hearing detection and intervention programs. American Journal of Audiology. 2000; 9:9-29. http://www.jcih.org/

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